Sebaceous gland tumors and systemic disease: a clinicopathologic analysis.

The association of sebaceous gland neoplasms with visceral carcinomas, first described by Muir and Torre, has become widely recognized. The purpose of our study was to determine the prevalence of malignancy, other associated diseases, and familial carcinoma in patients who had one or more cutaneous lesions within the spectrum of sebaceous adenoma, epithelioma, or carcinoma. Our histopathology files contained tissue from 59 such cases. Overlap between the categories was common, often precluding precise histologic classification. Of the 59 patients, 25 (42%) had one or more primary visceral malignancies. These totaled at least 49, of which 25 were colonic, 9 urogenital, 5 hematologic, 4 breast, and 6 miscellaneous. Fifteen patients (25%) had colonic polyps, most often multiple; and at least one primary carcinoma of the colon appeared in 10 of these, initially or later. Other less common findings included uterine fibroids, thyroid adenomas, and benign renal cysts. A family history of carcinoma was found in 72% of cases with visceral malignancy, most often of the colon and stomach. We conclude that sebaceous neoplasms form a histologic continuum, and strong associations with colonic polyps, internal malignancy, and a family history of carcinoma are apparent.

Necrobiotic xanthogranuloma with paraproteinemia. A review of 22 cases.

Cutaneous biopsy specimens from 22 patients showed the distinctive histopathologic pattern of necrobiotic xanthogranuloma within the dermis or subcutaneous tissue (or both). Twenty of the 22 patients had 1 or more serum protein abnormalities, consisting of an IgG monoclonal protein in 16, multiple myeloma in 3, cryoglobulinemia in 3, and an abnormal serum protein electrophoresis in 1. Cutaneous lesions were seen as discrete, slowly developing red nodules and plaques with a xanthomatized hue and a predilection for the face (periorbital region in particular), trunk, and extremities. Ulceration was a notable finding in 10 patients. Histologically, the dermis and lobules of subcutaneous tissue were involved with a granulomatous infiltrate containing bands of hyaline necrobiosis and bizarre foreign body, as well as Touton giant cells. Cholesterol clefts, lymphoid nodules with or without germinal centers, and foci of plasma cells were variable but significant features. Leukocyte monoclonal antibody studies in 6 patients demonstrated helper T cells within the granulomas. Electron microscopy in 3 cases showed lipid vacuoles in macrophages in the dermis and dendritic cells in the epidermis, and study confirmed this entity as a non-X histiocytosis. Pertinent laboratory findings, in addition to the serum protein abnormalities, included elevation of the erythrocyte sedimentation rate, leukopenia with absolute neutropenia, and decreased serum complement levels, as well as decreased levels of C1-esterase inhibitor in some patients. Thirteen of the 22 patients have survived, the mean duration being 9.5 years after the onset of cutaneous disease. While given to only a few patients in the current series, low-dose chemotherapy seems to induce a favorable response in both the cutaneous and the hematologic disease.

Cutaneous extravascular necrotizing granuloma and lymphocytic lymphoma.

A patient had clinical findings of asthma, peripheral eosinophilia, multisystem abnormalities, and cutaneous extravascular granulomas, consistent with a diagnosis of allergic granulomatosis. In addition, a well-differentiated lymphocytic lymphoma was diagnosed on the basis of bone marrow findings, and a monoclonal gammopathy was demonstrated by serum immunoelectrophoresis. Clinically, the skin lesions appeared as erythematous papules or nodules on the extremities, and some of them showed central ulceration. Histologically, there were dermal extravascular necrotizing granulomas embedded in a granulomatous and inflammatory cell infiltrate. These lesions bore a striking resemblance to those described by Churg and Strauss in patients with allergic granulomatosis, although tissue eosinophilia was significantly less prominent.

Gastrointestinal polyposis syndromes.

Well recognized cutaneointestinal syndromes in which colonic polyps are a constant and defining feature include Gardner's syndrome (familial adenomatous polyposis), Peutz-Jeghers syndrome, and Cronkhite-Canada syndrome. Colonic polyps are also found with increased frequency in Cowden's disease, the Muir-Torre syndrome, and neurofibromatosis. The Ruvalcaba-Myhre-Smith syndrome is a newly defined entity in this category. The relationship between acrochordons and colonic polyps remains a controversial issue, awaiting additional studies.

Histopathology of necrobiotic xanthogranuloma with paraproteinemia.

Fifty-three cutaneous biopsies from 22 patients with necrobiotic xanthogranuloma (NXG) were reviewed. One or more biopsies from each patient displayed a typical pattern of palisading histiocytic xanthogranuloma with bands of hyaline necrobiosis. Multiple foam cells and Touton giant cells were present, and atypical, bizarre-appearing foreign body giant cells were characteristic. Cholesterol clefts were found in 18 specimens, and lymphoid nodules were found in 24, 8 of which had germinal center formation. Twenty-one specimens contained foci of plasma cells, and these were located perivascularly, at the periphery of lymphoid nodules, and, as infiltrates, between dermal collagen bundles. Unique features were xanthogranulomatous panniculitis, often appearing as Touton cell panniculitis, and a rare but distinctive palisading cholesterol cleft granuloma. The cytoplasm of giant cells and histiocytes contained PAS-positive, diastase-resistant polysaccharide. Examination of 3 cases by electron microscopy revealed dendritic cells in the epidermis and vacuolated dermal histiocytes. S-100 antibody was negative in 3 cases. Leukocyte monoclonal antibody studies in 6 patients showed predominantly T-helper lymphocytes within the granuloma. NXG is a T-helper cell, non-X histiocytic granuloma with a distinctive histopathology.

Cutaneous immunofluorescence study of erythema multiforme: correlation with light microscopic patterns and etiologic agents.

Direct immunofluorescence microscopy was positive in 88% of forty-one skin biopsy specimens from thirty-four patients with the clinical diagnosis of erythema multiforme. The most common finding, present in 67% of positive specimens, was the cytoid body, or fluorescent keratinocyte, which stained most often with IgM (homogeneously) or with C3 (speckled). Other findings included basement membrane zone (BMZ) fluorescence, primarily with fibrinogen and C3, and vascular fluorescence, most commonly C3 in a granular pattern. Correlation of direct immunofluorescent and light microscopic findings revealed that (1) the fluorescent keratinocyte was prevalent only in epidermal and mixed patterns, correlating with the eosinophilic necrotic keratinocyte by light microscopy, and (2) vascular fluorescence was most prominent in dermal forms. Herpes simplex-associated erythema multiforme showed exclusively a mixed histologic pattern, whereas the drug-related form was primarily epidermal.

Juxta-clavicular beaded lines: a subepidermal proliferation of sebaceous gland elements.

Juxta-clavicular beaded lines typical arise at puberty as an asymptomatic, linear papular eruption involving the neck and supraclavicular areas. This entity appears to be more common in blacks than caucasians, and a female predominance has been noted. Microscopically, the papules noted clinically appear secondary to sebaceous gland hyperplasia, which is particularly prominent in the immediate subepidermal zone. Mild papillated epidermal hyperplasia may be observed. Whereas many regard this entity as an anatomic variant of normal, it may be confused clinically and histologically with a number of pathologic processes. A case of juxtaclavicular beaded lines in a 14-year-old caucasian girl is presented.

Cutaneous findings in hairy cell leukemia.

Hairy cell leukemia is a distinctive clinicopathologic entity that is histologically characterized by infiltration of the spleen, bone marrow, and blood by uniform mononuclear cells--"hairy cells." We reviewed the prevalent cutaneous findings, specific and nonspecific, in 113 cases of hairy cell leukemia. Only one patient had specific lesions (skin infiltration by leukemic cells). Nonspecific cutaneous findings included recurrent infections, ecchymoses, petechiae, pallor, drug reactions and reactions to transfusions, and nonherpetic ulcerations. Although leukemic infiltration of the skin in hairy cell leukemia is rare, cutaneous histopathologic features may be diagnostic, particularly when cytomorphologic examination is coupled with staining of a fresh specimen with tartrate-resistant acid phosphatase. Cutaneous and extracutaneous infections, often extensive and with opportunistic organisms, were the most common nonspecific findings and demonstrated severely deficient immunologic responsiveness. Associated malignant diseases, cutaneous and extracutaneous, were few in number and are of uncertain significance.

Paratesticular myxoma: an unusual benign intrascrotal neoplasm.

We have presented a case of paratesticular myxoma and have described the clinical history, findings on light microscopy and immunohistochemistry, and possible pathogenesis. Although primary paratesticular myxoma is a rare lesion, it should be considered in the differential diagnosis of intrascrotal mesenchymal tumors.